1/10. Severe menorrhagia due to Glanzmann thrombasthenia treated with hydrothermal ablation.Glanzmann thrombasthenia is a rare platelet disorder inherited as an autosomal recessive trait. Abnormal uterine bleeding is a common problem in women with the disease. Medical management may not always be effective and further treatment may be necessary. Two women underwent endometrial ablation with a continuous-flow circulating hydrothermal ablator. After follow-up of 12 and 18 months, both women remained without abnormal uterine bleeding.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
2/10. bernard-soulier syndrome in a Turkish family.This report describes the first Turkish family to be diagnosed with bernard-soulier syndrome. The family consists of nine members (two parents, three sons and four daughters). The parents were first cousins. The index case, a 22 year-old-man, had a history of haemorrhagic diathesis with thrombocytopenia, giant platelets in the peripheral blood smear and a prolonged bleeding time. Refractory idiopathic thrombocytopenic purpura had been diagnosed elsewhere and a splenectomy had been performed six months previously. ristocetin agglutination of platelets was defective and flow cytometry analysis of platelet membrane glycoprotein showed markedly reduced expression of glycoprotein lb (2.1%). bernard-soulier syndrome was diagnosed. Increased mean platelet volume was found in both parents, one son and three daughters. The other son and daughter were normal.- - - - - - - - - - ranking = 4keywords = platelet (Clic here for more details about this article) |
3/10. Uterine leiomyoma after embolization by means of gelatin sponge particles alone: report of a case with histopathologic features.We describe the histopathologic features of uterine leiomyoma after uterine artery embolization (UAE) in a 42-year-old woman. This patient, who was taking antiplatelet drugs for the treatment of cerebral disease, successfully underwent UAE using only gelatin sponge particles for a symptomatic uterine leiomyoma. Although menorrhagia improved moderately after the procedure, she underwent abdominal hysterectomy 11 months later because of recurrent uterine bleeding. Histopathology revealed that most of the area of the uterine leiomyoma was characterized by extensive coagulation necrosis, which support the positive result of the procedure. No significant abnormalities were noted in either the myometrium or endometrium, which also suggested that UAE using only gelatin sponge particles is an appropriate procedure to preserve the uterus. The histologic and radiologic features of this case are discussed. To the best of our knowledge, this is the first reported case of uterine leiomyoma after UAE using only gelatin sponge particles as a primary embolic agent.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
4/10. Acute myelogenous leukemia in an adult with thrombocytopenia with absent radii syndrome.Acute myelogenous leukemia with t(8;21)(q22;q22) developed in a 42-yr-old woman having thrombocytopenia with absent radii syndrome (tars). Standard induction and postremission therapies were safely administered. With each successive chemotherapy, the onset of platelet recovery was not delayed, but peak platelet counts were persistently suppressed. Nine months after achieving complete hematologic and cytogenetic remission, she remains severely thrombocytopenic (platelet count 6-12 x 109/L). She is, however, asymptomatic and transfusion independent. Of interest is the transient normalization of platelet count (rebound relative thrombocytosis). Our report and review of the literature suggests that tars, contrary to previous belief, may be associated with increased risk of acute leukemia.- - - - - - - - - - ranking = 4keywords = platelet (Clic here for more details about this article) |
5/10. menorrhagia due to a qualitative deficiency of plasminogen activator inhibitor-1: case report and literature review.A case is presented of a 26-year-old woman who was referred to the hematology clinic because of her report of a family history of plasminogen activator inhibitor-1 (PAI-1) deficiency. Since menarche, she had suffered from severe menorrhagia, but she had assumed that this was unrelated to her mother's history of repeated life-threatening bleeding. Her menorraghia was evident by using greater than 100 pads per period, bleeding as long as 4 continuous months, and even bleeding through her clothes despite using both tampons and pads. Evaluation with pelvic examination, endometrial biopsy, and pelvic ultrasound was unremarkable. medroxyprogesterone acetate treatment for her dysfunctional uterine bleeding was unsuccessful. Laboratory evaluation revealed iron deficiency anemia but otherwise normal platelets, bleeding time, prothrombin time, activated partial thromboplastin time, and vonWillebrand's studies. Despite any preconceptions, examination for a fibrinolytic defect ultimately demonstrated a PAI-1 antigen level of 11.4 ng/mL (4.0-43 ng/mL) and PAI-1 activity less than 5 AU/mL (5-37 AU/mL) and clinically supported a diagnosis of a hereditary, qualitative PAI-1 defect. She was treated with aminocaproic acid with return to relatively normal menses. Future treatment should also prevent excessive bleeding during trauma, surgery, or childbirth. Further evaluation of this patient and her family is planned and may help elucidate the important role of PAI-1 in the complicated balance between hemostasis and hemorrhage.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
6/10. Use of recombinant factor viia in the management of severe bleeding episodes in patients with bernard-soulier syndrome.bernard-soulier syndrome (BSS) is a rare congenital platelet disorder characterized by defective platelet adhesion and manifested by spontaneous and often profuse bleeding. Recombinant factor viia (rFVIIa) is a haemostatic agent licensed for the treatment of bleeding episodes in patients with haemophilia and inhibitors, which may represent a low-risk alternative to existing therapies in the management of patients with BSS. Here, we describe the use of rFVIIa for the treatment of three severe bleeding episodes in two patients with BSS. Data were extracted by automated searching of the international, internet-based registry http://www.haemostasis.com . Patient 1, a 24-year-old woman, was admitted with severe epistaxis and hypotension. The diagnosis of BSS was confirmed by macrothrombocytopenia, absence of ristocetin-induced platelet agglutination (RIPA) and absence of glycoprotein (GP) Ibalpha and IX on the platelet surface. Epsilon aminocaproic acid (EACA; two 50-mg/kg doses), packed red blood cells (PRBCs, 2 U) and platelets (30 U) failed to control the bleeding and, after 13 h, three bolus doses of rFVIIa (90 microg/kg body weight) and a third dose of EACA were administered; bleeding stopped after the third dose of rFVIIa. Patient 2, a 15-year-old girl, initially presented with severe menorrhagia. A lack of RIPA and severe deficiency of GPIbalpha on the platelet surface confirmed the diagnosis of BSS. EACA and fresh-frozen plasma did not control the haemorrhage, but two bolus doses of rFVIIa (98 microg/kg body weight) resulted in a marked decrease in bleeding. On second admission, patient 2 had severe epistaxis and mild menorrhagia. Two rFVIIa doses (98 and 122.5 microg/kg body weight) were given, and the bleeding stopped. No adverse events were reported in these cases. These three admissions highlight the potential of rFVIIa for the treatment of severe bleeds in patients with BSS.- - - - - - - - - - ranking = 6keywords = platelet (Clic here for more details about this article) |
7/10. Thrombocytopathia as a cause of menorrhagia. Two case reports.Thrombocytopathia is a condition in which the platelets have a qualitative rather than a quantitative defect. Two patients with thrombocytopathia who presented with menorrhagia are described.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
8/10. Unexplained menorrhagia and hematuria: a case report of Munchausen's syndrome by proxy.Unexplained menorrhagia and hematuria occurred in a 13-year-old female with a mild inherited platelet disorder who had never experienced prior bleeding as a result of this disorder. An intensive search revealed that this patient was receiving coumadin that was given by the mother. In addition, the mother drew large volumes of blood from the patient under the pretense of following a physician's orders. The mother also falsified the laboratory data to erase from her daughter's file a laboratory result documenting warfarin in the blood.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
9/10. Carotid artery thrombus associated with severe iron-deficiency anemia and thrombocytosis.BACKGROUND: Thrombus within the carotid artery usually occurs in vessels with severe atherosclerotic disease and may embolize to cause transient ischemic attacks and cerebral infarctions. The risk factors for carotid artery thrombus formation in the absence of atherosclerosis are not well characterized. A case series is presented that suggests an association of carotid artery thrombus with severe iron-deficiency anemia and thrombocytosis. CASE DESCRIPTIONS: We describe three women with severe iron-deficiency anemia and thrombocytosis secondary to menorrhagia who developed carotid artery thrombi. Thrombi were detected radiographically. The patients were treated with anticoagulation and antiplatelet therapy. In two patients, follow-up neuroimaging 10 to 14 days later demonstrated resolution of the thrombus and no identifiable vascular disease. CONCLUSIONS: Severe iron-deficiency anemia with thrombocytosis may be a risk factor for carotid artery thrombus formation. Medical management with anticoagulation and antiplatelet therapy is a reasonable approach for these patients while the thrombus resolves.- - - - - - - - - - ranking = 2keywords = platelet (Clic here for more details about this article) |
10/10. Severe juvenile vaginal bleeding due to Glanzmann's thrombasthenia: case report and review of the literature.Glanzmann's thrombasthenia is a rare inherited hematological disorder defined by deficiency or abnormality of the glycoprotein (GP) IIb-IIIa complex. Presenting symptoms are hemorrhagic events, mainly epistaxis, purpura, or menorrhagia. We describe the clinical course and management of a 14-year-old girl with Glanzmann's thrombasthenia and severe menorrhagia. Following treatment with 20 U of packed red blood cells, 37 U of platelets, 7 U of fresh frozen plasma, cryoprecipitate, intravenous estrogens, and methylergotrine maleate with no improvement, the uterine cavity was packed for 48 hr. This unusual procedure halted the bleeding and avoided the necessity for a hysterectomy. When treating acute menorrhagia in patients with Glanzmann's thrombasthenia, the physician should be familiar with the characteristics and all treatment modalities for this disorder.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
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